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Microscopic Polyangiitis: Atypical Presentation With Extensive Small Bowel Necrosis, Diffuse Alveolar Hemorrhage, and Renal Failure FREE TO VIEW

Justin Segraves, MD; Vivek Iyer, MD
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Mayo Clinic, Rochester, MN

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A197. doi:10.1016/j.chest.2016.02.204
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SESSION TITLE: Diffuse Lung Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Microscopic polyangiitis is one of the pulmonary-renal vasculitides syndromes associated with MPO and p -ANCA antibodies which can cause diffuse alveolar hemorrhage (DAH), acute kidney injury (AKI), and rarely mesenteric ischemia. We present a case wherein all of these complications occurred which required maximal support including plasma exchange (PLEX).

CASE PRESENTATION: A 56 year old male patient presented to an outside hospital with a several month history of constitutional symptoms and was initially diagnosed with acute pancreatitis. He subsequently developed respiratory failure requiring intubation and transfer to our hospital. A bronchoscopy revealed a progressive bloody return indicating DAH. He developed AKI with a peak creatinine of 6.5 mg/dL. Urinalysis showed proteinuria and RBC casts. Antibodies were positive for MPO and p-ANCA. The kidney biopsy was consistent with ANCA associated vasculitis. High dose steroids, rituximab, PLEX, and hemodialysis were initiated with considerable improvement allowing for extubation. However, a few days later he developed acute abdominal distention and respiratory distress requiring re-intubation. An abdominal CT suggested extensive small bowel ischemia. An emergent laparotomy revealed necrotic bowel requiring removal of 60% of the small bowel and an end ileostomy. He was ultimately discharged after a prolonged hospital stay including 2 weeks in the ICU. He required hemodialysis for one month and also needed total parenteral nutrition for short-gut syndrome.

DISCUSSION: Our case highlights the severe multi-systemic involvement rarely seen in its entirety in microscopic polyangiitis. What makes our case unique is the atypical presentation with initial pancreatitis followed by extensive small bowel necrosis, AKI, and DAH.

CONCLUSIONS: Microscopic polyangitis can present in an atypical fashion with life threatening complications. Prompt diagnosis and management with high dose IV steroids and either cyclophosphamide or rituximab1 is required. PLEX is typically used in severe disease and its role is under investigation in an ongoing clinical trial (PLEXIVAS). 2, 3

Reference #1: Beck L, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. Am J Kidney Dis 2013;62:403.

Reference #2: Walsh M, et al. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. Am J Kidney Dis 2011;57:566.

Reference #3: Casian A, Jayne D. Plasma exchange in the treatment of Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis. Current Opinion in Rheumatology 2011, 23:12-17.

DISCLOSURE: The following authors have nothing to disclose: Justin Segraves, Vivek Iyer

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